Pathophysiology of exertional death associated with sickle cell trait: Can we make a parallel with vaso-occlusion mechanisms in sickle cell disease?

摘要

Whether sickle cell trait (SCT) carriers are at greater risk for exertional death has been a subject of intense debate. Large epidemiological studies in warfighters and athletes clearly show an increased risk for exercise-related sudden death in SCT carriers compared with non-SCT carriers.The pathogenesis of exertional death in SCT carriers is unknown. However, the mechanisms initiating these adverse events could be parallel to the ones involved in the vaso-occlusion crises in sickle cell disease (SCD). Vaso-occlusion in patients with SCD involves haemorheological alterations, inflammation, vascular-adhesion processes and endothelial dys-function.